背景:视神经脊髓炎谱系障碍(NMOSD)是一种炎症性自身免疫性疾病,具有高复发和残疾风险,治疗目标是无复发状态。后区域(AP)是NMOSD最常见的涉及区域之一,这可能在NMOSD的发病机制和临床异质性中具有特殊意义。我们的研究是探讨AP发病NMOSD患者的临床和复发特征。
方法:对根据2015年IPND标准确定的166例AQP4-IgG血清阳性NMOSD患者进行了一项回顾性研究。根据初始发作位置将患者分为AP发作(APO-NMOSD)组和非AP发作(NAPO-NMOSD)组。比较两组患者临床特征及复发差异。
结果:APO-NMOSD组和NAPO-NMOSD组的人口比率为24:142。APO-NMOSD患者较年轻(34.6yVS42.3y,P=0.013),在第一次发作(0.7VS4.2,p=0.028)和最后一次随访(1.9VS3.3,p=0.001)时EDSS较低,在第一次发作时更可能有多核心病变(33.3%VS9.2%,P=0.001)。此外,他们的年复发率较高(0.4±0.28VS0.19±0.25,P=0.012)。在自然过程中,没有免疫治疗的NMOSD患者,与NAPO-NMOSD相比,APO-NMSOD的首次复发时间短(P<0.001),年复发率高(0.31±0.22VS0.16±0.26,P=0.038)。与视神经炎发作-NMOSD(HR2.641,95%CI1.427-4.887,p=0.002)和脊髓炎发作-NMOSD组(HR3.593,95%CI1.736-7.438,p=0.001)相比,APO-NMOSD组首次复发的风险更高。与NAPO-NMOSD相比,APO-NMOSD脑干复发的可能性更高(28.6%vs.4.7%,p<0.001)在第一次复发期间,而NAPO-NMOSD更容易受累于视神经(10.7%vs.41.1%,p=0.01)。
结论:AQP4-IgG血清阳性的NMOSD患者发病年龄较轻,复发风险较高。临床医生应注意NMOSD中AP的损伤,因为这表明有潜在的复发风险。
背景:回顾性注册。
BACKGROUND: Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory autoimmune disease with high risk of recurrence and disability, the treatment goal is a recurrence free state. Area postrema (AP) is one of the most common involved area of NMOSD, which may have a particular significance in the pathogenesis of NMOSD and clinical heterogeneity. Our study is to investigate the clinical and recurrent characteristics AP onset NMOSD patients.
METHODS: A retrospective study was done in a cohort of 166 AQP4-IgG seropositive NMOSD patients which were identified by the 2015 IPND criteria. The patients were divided into AP onset (APO-NMOSD) group and non-AP onset (NAPO-NMOSD) group based on the initial episode location. Clinical features and recurrence differences of two groups were compared.
RESULTS: The APO-NMOSD group and NAPO-NMOSD group had a population ratio of 24:142. APO-NMOSD patients were younger (34.6y VS 42.3y, P = 0.013), had lower EDSS at first episode (0.7 VS 4.2, p = 0.028) and last follow up (1.9 VS 3.3, p = 0.001), more likely to have multi-core lesions at the first attack (33.3% VS 9.2%, P = 0.001). Also, they had a higher annual recurrence rate (0.4 ± 0.28 VS 0.19 ± 0.25, P = 0.012). In natural course NMOSD patients without immunotherapy, APO-NMSOD had a shorter time of first relapse (P < 0.001) and higher annual recurrence rate (0.31 ± 0.22 VS 0.16 ± 0.26, P = 0.038) than NAPO-NMOSD. APO-NMOSD group also have a higher risk of having the first relapsing compared to optic neuritis onset-NMOSD (HR 2.641, 95% CI 1.427-4.887, p = 0.002) and myelitis onset-NMOSD group (HR 3.593, 95% CI 1.736-7.438, p = 0.001). Compared to NAPO-NMOSD, APO-NMOSD has a higher likelihood of brainstem recurrence (28.6% vs. 4.7%, p<0.001) during the first recurrence, while NAPO-NMOSD is more susceptible to optic nerve involvement (10.7% vs. 41.1%, p = 0.01).
CONCLUSIONS: AQP4-IgG seropositive NMOSD patients with AP onset are youngers and have higher risk of recurrence. Clinicians should pay attention to AP damage in NMOSD, as it indicates a potential risk of recurrence.
BACKGROUND: Retrospectively registered.